Friday, April 29, 2016

Wishing there was an answer/solution. Headaches, Heart Rhythm Appt, etc. .. At a loss

I have to start this by apologizing for any errors in this especially typos/ep errors as vision has decided it's going to be crappy and so trying to read this even with the bifocal part of my lenses is something short of aggravating. I swear I don't know what it is with shunts other than I know my body does this slloooowwww unwind (for lack of better word/way to describe it) in which I go from feeling phenomenal (like really good) especially after Nov's surgery, to put back in the 2nd shunt - (I keep thinking about my Sister and I standing out in her front yard, she was putting up lights or something in their tree not long after that surgery and we where talking about this all and she asked if I thought this might be the time that my feeling 'really, really good' actually lasted. I don't know what I said in response but am sure it was something to the affect I wasn't focusing on how long it lasted but even if we got 6mo or no matter how long we got it was AMAZING. Sure there was a wk where things where rocky prior to the 3rd OHS, my body just being stupid but then I basically went in to the OHS feeling great from a shunt/headache standpt.
It seems anytime we would/did get the shunts to a really good place some stupid thing came up that affected them (The initial surgery with Dr.Bragg where the single shunt was working better after she revised it but then fluid was also making it's own pocket in my back/spine (pseudomeningele I think is what it was called) which allowed more fluid to drain, when we'd to the external drains, when we added the 2nd shunt, times like 1.5yrs ago or so ago after a revision, before fluid started building up in lung space and wasn't absorbing I remember how happy I was at feeling so good (and how disappointed I was when I wasn't feeling as good again!).
Anyways this post isn't to vent about the past though, sorry. I can't change what I cant change but do I wish I'd magically go in to Dr.Bragg's office, before she leaves towards the end of May (around the 21st) and she'd have some idea and something she could do to help. It sounds ridiculous but at this point I wouldn't even care if it where a flipping surgery bc I feel so blessedly bad so much of the day. It is only by this time of day I finally feel better which makes for having to force myself to go to bed atleast by midnight despite knowing i'll then feel worse again. UGH!!!! Have I said I hate this? Why couldn't it be simple? I can't even say how much I've prayed for us to find an answer before Dr.Bragg moves. =/
Heart Rhythm Appt On a different note, I followed up with Dr.Kovach, Heart Rhythm / Cardiology today (he does Heart Rhythm works with my Cardiologist on my Team) and biggest thing was given Heart rate has been so high (I don't make a habit of tracking it but for information's sake I do record it 1-2 times a day on an app on my phone so we have an idea how the meds are working). Anyways despite our increasing the Ivabradine (Corlanor) to 10mgs, which is a non standard dose (not studied in the original clinical trials for this med) Heart rate has stayed high and this wk routinely much higher for longer periods (hours) average ranging from high 90s but often was 140s for wide periods this wk. Why? Who knows, that would be the answer we'd probably all love to know!? - That and a light bulb that blinks with a easy solution! Ahh but there again, that same light bulb moment could help us with the damn shunts?!!?!?!?!
Anyways because the Heart Rhythm Study/Ablation is risky Dr.Kovach would like to try a few other meds/med classes, 1 at a time which i'll take in addition to what I already take (but have been on for quite a long period, other than the Lisinopril, an Ace Inhibitor that was started right after this last OHS in Jan.). So the grand decision was to go back to a different beta blocker, this 1 being 1 Dr.Earing tried some yrs ago, though it didn't have much if any success then it also had virtually no side effects (a miracle for the BB class of heart meds). The heart rhythm issues also are likely different now than they where a few yrs ago as that if I remember right was pre-any of the heart valve surgeries and after 3 OHS the wiring in the heart can be affected.
Thinking ahead to if that doesn't work/help he (Dr.Kovach) thought he may try a Calcium Channel blocker (CCB) and gave 2 med names, 1 of which we (Dr.Earing and I) had used for some time a few yrs back. I can't remember what the difference is in CCB class of meds vs the Beta blockers (BB's) but they must each work differently on the heart? At one point I'm sure I knew! Should that all fail we'll revisit the Heart Rhythm Study/Sinus node ablation so time will tell..
Dr.Bragg always asks me about the heart stuff, Cardiology (Dr.Earing and Dr.Kovach both) always ask me about the shunt stuff so today I was kind of filling Dr.Kovach and his Nurse in on the seeming slow downward slope the shunts seem to be in, how it's slowly getting worse but like many times is in the period where it's as clear as unclear can get and basically I think it just comes down to I have to wait till 1 or both fail completely which they will (I know that sounds very negative but it's experience and history that make me know it will happen). In the interim I keep praying Dr.Bragg could have some divine idea or we could figure this out before she leaves but honestly I am losing hope, not in her but just in general that I'm going to be feeling better before she leaves. This is soo hard to think about so I try not to but not exactly easy to do whe nmuch of the day my head feel like it is in the clouds, filled with thick clouds. I guess I just keep praying in the interim I can keep dealing w it, not a lot of other choice, right? All these headaches and in turn nausea in the night and morning means dealing with very unstable INR, this in turn requiring the self injections (they suck but thankfully if I give them quickly and pinch the area of my stomach I inject it isn't very uncomfortable). INR being unstable and the injections in turn require and frequent Lab draws to watch INR level which shows if blood is where it needs to be to be most safe for the 2 valves to keep from clotting.
We'd gotten it back to a 'close enough to normal' range late last wk I was able to stop the injections but then was massively low again on Tues so back on the lovenox on top of higher Coumadin dosing. Range was up a little today so continue injections 2x day and recheck Monday at CHW prior to ERT next door.
Not to be negative but is shitty, what else is there to say? I had to stop at a drs office today to pick up scripts and in chatting the Secretary made the comment 'Youre always so positive' - honestly I appreciate that but lately I don't feel like it's all that true!? I feel pretty sarcastic lately, not at anyone but just as a coping mechanism I think while feeling so bad so often. I am always thankful I generally don't see people till after I've been up for several hours and most often if I have a meeting (Sunday School Teachers for instance) or Cardiac Rehab (which I've missed a few the past wkS) it's not till later in the day.
As I said to my Mom yesterday there are times I don't even want to be around myself, I feel like a grouch much of the day! I apparently have an ability to hide that from others which I guess is good!?! I think that same ability is what makes it fairly easy to hide from many how I truly feel as most people aren't really looking/paying attention and I often let other people do the talking and just chime in a bit vs when I feel really, really good I kind of talk a lot, lol. I sometimes wish I was better like some people are at being reallllyyy honest about how I am feeling though, does that help some people get answers sooner? I'm sure it's a question I've asked her before.. and no real answer.
But anyways going to sign off, I see Dr.Bragg late next wk. I probably wrote about it here but i've decided on the new (coming) Peds Nrsgn but really have no idea what I need to do if anything so he is aware of my history (or will she talk to him or both of us when I 1st see him could him and I conference her)? Ugh, have I ever said I hate this kind of thing? I've never really been a fan of mtng new docs, nothing against them just feel like well maybe like I am hitting new docs with a tsunami and few really, truly want a big challenge? I don't know really, but what I do know is in the past 12yrs (5yrs with Dr.Bragg in May ironically) every single time I've had to switch a Specialist no matter the reason I've hated it. I've met some of the best docs on my Team from switching and don't regret any of the switches I've made but I think it scares me in a way b/c your mtng someone you have to hope will care enough and be open enough to want to truly help. Your hoping the new doc is the type that makes their own decisions vs 'what the textbook says' (b/c MPS and especially my issues and MPS aren't in no manual be it Hydrocephalus, MPS or anywhere... -
Deep breathes, right? I'm trying, sometimes easier than others and failing miserably at not worrying about it (as I said to someone else or perhaps it was to someone's ques. about something on fb, I think I've worried more in the past 2mo than I have in 32yrs prior! Not really that awesome.
I guess I just have to hope Dr.Bragg will be amenable to us reaching out to her both this new Nrsgn when he comes Mid-Aug. and my other Specialists if need be and pray she really will be ok with that and will stay involved in some capacity. I'm sure it's obvious but I worry that someone like her who knows my oddities (MPS wise, lol) will still help us till the new guy, if god willing he turns out to be ok gets a handle on it all. Without a doubt that is my biggest worry with her leaving is going back to no one that will help the less than straightfwd issues and have patience and be willing to work with my Team at large.

Enough stressing though, right? Sorry!
See below for trip to Boston but thanks for stopping by, Erica

I did go to Boston, which despite not feeling the best was a lot of fun, was really nice weather and I had plenty of downtime so worked OK. I definitely didn't feel great all of the time but do not regret going.
I feel like MPS and how I feel already impacts so much of my life so I try to do the little things I can to make trips like that more manageable and in turn keep living despite feeling bad.
The marathon was great, the weather was finally nice (kind of bad for our runners) and a decent day in general. My flight home wasn't till 7:30 so worked out nicely being able to see Sarah, my runner after she finished the race. She did amazing in my opinion especially this being the 1st marathon she's ever run! So cool!!!
I got to meet her kids and husband with Sarah at the Running4Rare Team dinner at Harvard that Sat night and immediately feel in love with her 3 kiddos. They where awesome!
Sarah and I went to the Marathon expo Sun early afternoon which was nice, it at least allowed my headaches (pressure) to be better under control from being up quite a while! I took the train from Hopkinton to Boston (roughly an 45min-hour ride) to meet Sarah and only managed to screw up the train schedule a couple times, something of a miracle these days! Sarah and Ed (Ed is 1 of the 2 friends I was staying with, he and his wife Anne and their 2 kids are also on the RfRD Team and represent another rare disorder CHI), they all helped with planning times and mtng spots so that of course helped immensely! which was miraculous though Sarah helped make sure I got that all ok!

Sarah was my running partner this yr, which it turned out was a pretty good match as we have a lot in common despite different 'roles' in the rare disease world (Sarah as a Rare Mom, myself as Patient and each of us dealing with 2 different ultra rare disorders).

I think as Sarah put it we knew we'd get along well when she saw my post after the OHS back in Jan., something to do with how gross hospital floors are and she (lol) gets the same creeps I do about them (and same desire to just get out a broom)! We've had many a laugh about that =)
Anyways so much for signing off above, if you would please say a prayer Dr.Bragg might have some idea how to help before she leaves. I hate surgery but damned I'd even take that in a heartbeat if it meant feeling better. I HATE THIS!!!!!!

Friday, April 8, 2016

"Your body reads the 1st line.... ( Cardiolody/Heart Rhythm Appts )..... Makes it's own rules"... -

"Your body reads the 1st line in the manual and makes its own rules" - Dr.Bragg
Why that quote/title you you ask? (Which I came across in an old draft on here I'd never finished while clearing out things I didn't need) but it's a quote I distinctly remember Dr.Bragg saying albeit she was saying it at the time to make me laugh I believe (was after an unusual shunt infection presentation and catch, per the unfinished entry where she and her 1 NP had just come by my rm and gave me the news of the unexpected infection (we all thought it was gallbladder related, General Surgery was overseeing everything but Dr.Bragg in her usual nature was staying involved and doing what she could - i'll miss that about her. She always knew what was going on and knew what to do!
Needless she was right then and it applies today, to Cardiology /Heart Ryhthm drs. and seemingly to today's appt! I guess i'll just update on what we all talked about and where things are at? I haven't updated since last wk sometime so I re-checked INR on Monday and that was realllyyyyy low (1.2 I believe off hand) which is basically at a level one would be at if they weren't needing to be on Coumadin in the 1st place.
There where several days last wk I was sick, threw up in the mornings and although it didn't occur to me at the time I suspect/am guessing the Coumadin which I take in the morning likely wasn't dissolved/absorbed yet and likely came up with that funness (my own word?)? I don't really know. Needless to say when Cardiology called with the result Monday they where concerned b/c when there is a mitral valve and aortic valve the risk of clots when so low is apparently greatly increased. They called in Lovenox (Enoxaperin) injections to initially do for 2 days and increase Coumadin to 10mgs times 2days, recheck before today's Appt. Labs done before today's Cardiology and Heart Rhythm Appts. show INR was 1.4 so not an great increase. This in turn means stay on the self injections 2x per day till Mon, re-check Mon.

Cardiology / Electrophysiology (Heart Ehythm) Appts:
Today's Cardiology/Heart Rhythm Appts went ok, nothing to earth shattering (do I say that a lot?) though a med dose change and if that doesn't work possible heart procedure to consider.
Med wise although it's not a commonly used dose (the med is fairly new, having been FDA approved last Spring, about a year ago) but we are going to trial adjusting the dose from 7.5mgs to 10mgs. He isn't sure this will work as what little evidence there is on the med shows statistical difference in those pts. taking 7.5mgs vs those few taking 10mgs is limited (not significant). Dr.Kovach commented something about with this particular med it seems evidence has shown at this highest dose the body seems to more often break the higher doses down vs being able to process and use it effectively.
He did comment though that each person responds differently and was worth our trying. He seemed to feel since it's a med I've had the least amount of (really no) side effects with and fairly decent heart rate improvement, prior to the Mitral valve replacement it is worth trying the increase. I think our average heart rate pre-MVR was around 80bpm prior the surgery after starting the Ivabradine though we also had several other meds to help. The other meds (Digoxin, Lisinopril (added right after surgery due to slightly decreased heart muscle function) and the Ivabradine as well as 2 high dose meds for fluid for prevent heart-lung fluid overload and to help overall heart muscle function and to help heart rate if I remember right, prior to Jan.
Unknown, Potential Heart Cath-Ablation

It really is an unknown if this will help but we all feel it is worth trying and Dr.Earing (my Cardiologist) when he stopped by felt the same. It is doesn't work or any problems Dr.Kovach mentiond potentially trying a different beta blocker or calcium channel blocker we've tried in the past as the nature of the heart rate problem can change after a Open Heart surgery. We'd more than likely still keep the Ivabradine and the other meds I am on, at the current doses as these are used to help heart muscle function better and likely have some affect on heart rate now).
The other thing he brought up was he said he may consider and asked my thought re going back in via a heart cath (catheters threaded typically via the groin) and doing a heart rhythm study known as a 'Sinus Node Ablation' where they thread catheter(s) around the sinus node to try and ablate around the sinus node and try to get this to "chill out" (lol, that made me laugh seriously when he said that). In doing this approach we would bypass other "drug cocktails" and try this lesser used procedure which does carry the potential risk of causing to slow of heart.
- I didn't think to ask what degree the risk might be (causing to slow of heart rate or if that is just an unknown) and thus need for a Pacemaker. This in turn I also did not think to ask can a Pacer even be done when there is programmable shunts in place given the shunts have to be reset by magnets and I believe Pacemakers do to? Something i'll have to ask Dr.Bragg about out of curiosity next wk. For now we'll pray the new dose perhaps helps, never know!? I seem to do the opposite of what's the norm so maybe this to will be a abnormal, my normal given many seem to not have HR change perhaps mine will again? Cant hurt to pray and hope! =)
I admittedly can't entirely remember what Dr.Earing and I talked about (I got about 3 or 4hrs of sleep Weda night, fell asleep around 10-11 and woke around 2, couldn't fall back asleep. I remember the stuff from Dr.Kocach's appt as I wrote notes but Dr.Earing came in shortly after and he just wanted to check in. In Dr.Earing's case was more to review what Dr.Kovach talked about I think and more importantly go over the to low of INR and figure out a plan there. Instead of stopping the Lovenox after 2 days i'll stay on it now through Monday in addition to the increased Coumadin dose and then re-test Labs Monday. We'll go from there.
I'll see the 2 of them either together or separately again in 3wks to review this med dose adjustment and overall. 1 other thing I remember Dr.Kovach pondering over was other things that could be affecting heart rate but like Dr.Earing he ticked those off and essentially said based off history he doesn't believe those are causes.
Some things he to mentioned (he always asks how the shunts are, have I had any shunt surgeries since I've last seen them and usually knows I've not) where 1. pain but he to felt that was very unlikely. 2. Shunts but he said pretty much as Dr.E has that we've had numerous shunt surgeries in the period we've been dealing with more difficult/higher heart rates and yet no matter when really good or when problematic heart rate variable doesn't seem to change. So he doesn't feel that's likely affecting it. He commented something along the line 'could it be some unusual problem in you not traditional described for MPS but there to he couldn't say and wasn't sure any one could. I don't think we'll ever really know the answer to this question really. Sooner or later we'll sort it out.
Headed to Boston, Boston Marathon / Running for Rare Team, Genetics Appt (Boston Children's/Dr.Bodamer), seeing friends -

In the interim I see Dr.Bragg next wk and fly to Boston that next (Thurs) morning. I am hoping I have a little more energy by then or at the very least I am hoping I get enough relief to feel OK for my flight on Thurs morning. I fly out of Milw., an airport I don't use quite as much these days (but am familiar with) and have a non stop flight to Boston. I'll see my Genetics dr. late the next day. ---
On a side note, I signed up/was interviewed for a speakers bureau position with Genzyme, something Patients have to do a simple interview for and then that gets reviewed and if accepted by the Genzyme folks you go though a training process with your local Patient Liaison. I'll do the training part in a few wks. In the interim unrelated to that i'll see a bunch of friends and my Insur. Case Manager from Genzyme while I am out there next wk which is always nice! To top that all of i'll get to finally meet my RfRD Runner, Sarah and her family! Exhausting, a little daunting (due to not feeling the best) but really looking fwd to it all! Thankfully my flight back Monday of Marathon day is that evening so less stress this yr. trying to see my runner and still get to the airport on time! I also have a non stop flight on the way home (yippee!!). =)
Will try to update soon, have a few questions for Dr.Bragg next wk so will update about those perhaps after I get back from the Marathon and Boston - I am super excited to see the many friends I have out there including the friends I am staying Fri-Monday with (Thurs i'll stay overnight in a hotel, close to BCH where Dr.Bodamer is). =)

Running for Rare - Boston Marathon Shirt TEAM MPS

Lovenox (Enoxaperin) Injections -

Easter - Grandpa, Bella, Zander (he was by his Dad, taken the next wknd)